Long-Term Follow-Up of a Pediatric Cohort With Short QT Syndrome

Villafañe, Juan; Atallah, Joseph; Gollob, Michael H.; Maury, Philippe; Wolpert, Christian; Gebauer, Roman; Watanabe, Hiroshi; Horie, Minoru; Anttonen, Olli; Kannankeril, Prince J.; Faulknier, Brett; Bleiz, Jorge; Makiyama, Takeru; Shimizu, Wataru; Hamilton, Robert M.; Young, Ming‐Lon

doi:10.1016/j.jacc.2012.12.025

Cited by 89 publications

(32 citation statements)

References 26 publications

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“…46 Sixteen patients (84%) had a familial or personal history of cardiac arrest. A gene mutation associated with short-QT syndrome was identified in 5 of 21 probands (24%).…”

Section: Sudden Cardiac Arrestmentioning

confidence: 99%

Heart Disease and Stroke Statistics—2017 Update: A Report From the American Heart Association

Benjamin¹,

Blaha²,

Chiuve³

et al. 2017

Circulation

7,718

5,040

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“…46 Sixteen patients (84%) had a familial or personal history of cardiac arrest. A gene mutation associated with short-QT syndrome was identified in 5 of 21 probands (24%).…”

Section: Sudden Cardiac Arrestmentioning

confidence: 99%

Heart Disease and Stroke Statistics—2017 Update: A Report From the American Heart Association

Benjamin¹,

Blaha²,

Chiuve³

et al. 2017

Circulation

7,718

5,040

View full text Add to dashboard Cite

“…These findings may reflect similar manifestations of aberrations in atrial refractoriness. Villafañe et al 19 found that 24% with short-QT syndrome experienced aborted sudden cardiac death during follow-up. In our study, no patients were documented to have experienced aborted sudden cardiac death or ventricular arrhythmia during the follow-up period.…”

Section: Discussionmentioning

confidence: 99%

Short QT Interval Prevalence and Clinical Outcomes in a Pediatric Population

Guerrier

Kwiatkowski

Czosek

et al. 2015

Circ: Arrhythmia and Electrophysiology

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Background-Risk associated with short QT interval has recently received recognition. European studies suggest a prevalence of 0.02% to 0.1% in the adult population, but similar studies in pediatric patients are limited. We sought to determine the prevalence of short QT interval in a pediatric population and associated clinical characteristics and outcomes. Methods and Results-Retrospective review of an ECG database at a single pediatric institution. The database was queried for ECGs on patients ≤21 years with electronically measured QTc of 140 to 340 ms. Patients with QTc of 140 to 340 ms confirmed by a pediatric electrophysiologist were identified for chart review for associated clinical characteristics, symptoms, and outcome. Patients with and without symptoms were compared in an attempt to identify variables associated with outcome. The query included 272 504 ECGs on 99 380 unique patients. Forty-five patients (35 men, 76%) had QTc ≤340 ms, for a prevalence of 0.05%. Median age was 15 years (interquartile range, 2-17), median QT 330 ms (interquartile range, 280-360), and median QTc 323 ms (IQR,(313)(314)(315)(316)(317)(318)(319)(320)(321)(322)(323)(324)(325)(326)(327)(328)(329)(330)(331)(332). Women had significantly shorter QTc compared with men (312 versus 323 ms; P=0.03). Two deaths were noted in chart review-one from respiratory failure and the second of unknown pathogenesis in a patient with dilated cardiomyopathy. Conclusions-Short QT interval was a rare finding in this pediatric population, with a prevalence of 0.05%. Male predominance was identified, although the median QT interval was significantly shorter in women. There seem to be no unifying clinical characteristics for this pediatric patient cohort with short QT interval. (Circ Arrhythm Electrophysiol.

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“…In a study by Giustetto et al [6], 3 children with SQTS presented with aborted sudden cardiac death or syncope during infancy and 2 other neonates in families with SQTS had died suddenly. Villafañe et al [15] reported symptoms in 14 (56%) of 25 SQTS pediatric patients, including aborted sudden cardiac death in 6 (24%). Arrhythmias included ventricular fibrillation in 6 and AF in 4 patients of the pediatric cohort.…”

Section: Discussionmentioning

confidence: 99%

Short QT Syndrome Manifesting with Neonatal Atrial Fibrillation and Bradycardia

Villafañe

Fischbach²,

Gebauer³

2014

Cardiology

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Objectives: Atrial fibrillation (AF) is rare during childhood and usually associated with other cardiovascular pathology. In lone AF, the ventricular response rate is usually rapid. We sought to describe a subset of children who present with early-onset AF and a slow ventricular response rate who were found to have the short QT syndrome (SQTS). Methods: Using a MEDLINE/PubMed search, children with AF, a structurally normal heart and bradycardia were identified. Demographics, clinical presentation, electrocardiographic (ECG) findings, electrophysiologic testing, genetic analysis and follow-up assessment were collected on each child for analysis. Results: Four children were identified in the literature and combined with 2 other children followed by the authors. All had a short QT interval and those who were tested were found to have a gain-of-function mutation in the KCNQ1 gene. Conclusions: We describe a subclass of children with SQTS who present with AF and a slow ventricular response. Medical therapy has not been effective in maintaining sinus rhythm. The long-term outcome remains unknown for these children. This condition may present in utero as persistent bradycardia with postnatal ECG showing a very short QT interval.

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Long-Term Follow-Up of a Pediatric Cohort With Short QT Syndrome

Cited by 89 publications

References 26 publications

Heart Disease and Stroke Statistics—2017 Update: A Report From the American Heart Association

Heart Disease and Stroke Statistics—2017 Update: A Report From the American Heart Association

Short QT Interval Prevalence and Clinical Outcomes in a Pediatric Population

Short QT Syndrome Manifesting with Neonatal Atrial Fibrillation and Bradycardia

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